2010美国PWSA帕-魏二氏综合症患儿脊柱侧弯的监测和治疗指南

Children with Prader Willi Syndrome have an incidence of developing scoliosis at rates between 40 -90%. Approximately15% of children with Prader Willi Syndrome will develop severe or significant curves, requiring bracing or surgery. The earlier the curve is detected, the better the  possibilities for treating the curve with casting or bracing.

There are two peak ages for scoliosis presentation in children with PWS. Under the age of 4 years, most of the curves are C-shaped, and are most likely related to the hypotonia. The second peak, centered around 10 years of age, typically is the more common idiopathic S-shaped curve. Fifteen percent of curves diagnosed before 4 years of age subsequently required surgical treatment, 41% of curves diagnosed after 4 years of age required surgical correction, as per a PWSA-USA survey of membership. Spinal deformities in children with PWS are often diagnosed late. This delay appears to be due to unique characteristics of spinal deformities in children with PWS, rather than the presence of obesity. Fewer children with PWS now develop obesity, and often the curves are diagnosed prior to the onset of obesity. What seems to be the more important factor is that spinal deformities in children with PWS have less vertebral rotation than seen in other children with scoliosis curves of a similar size. Vertebral rotation causes the asymmetry of the chest wall seen during forward bending, usually the first sign of scoliosis. Therefore, the child with PWS may have a moderate curve radiographically, but only mild findings clinically. For that reason, there should be a much lower threshold for working up clinical findings in children with PWS, compared to otherwise unaffected children.